dc.contributor.author | Bastard, Paul | |
dc.contributor.author | Orlova, Elizaveta | |
dc.contributor.author | Sozaeva, Leila S. | |
dc.contributor.author | Lévy, Romain | |
dc.contributor.author | James, Alyssa | |
dc.contributor.author | Schmitt, Monica M | |
dc.contributor.author | Ochoa, Sebastian | |
dc.contributor.author | Kareva, Maria | |
dc.contributor.author | Rodina, Yulia | |
dc.contributor.author | Gervais, Adrian | |
dc.contributor.author | Le Voyer, Tom | |
dc.contributor.author | Rosain, Jérémie | |
dc.contributor.author | Philippot, Quentin | |
dc.contributor.author | Neehus, Anna-Lena | |
dc.contributor.author | Shaw, Elana | |
dc.contributor.author | Migaud, Mélanie | |
dc.contributor.author | Bizien, Lucy | |
dc.contributor.author | Ekwall, Olov | |
dc.contributor.author | Berg, Stefan | |
dc.contributor.author | Beccuti, Guglielmo | |
dc.contributor.author | Ghizzoni, Lucia | |
dc.contributor.author | Thiriez, Gerard | |
dc.contributor.author | Pavot, Arthur | |
dc.contributor.author | Goujard, Cecile | |
dc.contributor.author | Fremond, Marie-Louise | |
dc.contributor.author | Carter, Edwin | |
dc.contributor.author | Rothenbuhler, Anya | |
dc.contributor.author | Linglart, Agnès | |
dc.contributor.author | Mignot, Brigite | |
dc.contributor.author | Comte, Aurelie | |
dc.contributor.author | Cheikh, Nathalie | |
dc.contributor.author | Hermine, Olivier | |
dc.contributor.author | Breivik, Lars Ertesvåg | |
dc.contributor.author | Husebye, Eystein Sverre | |
dc.contributor.author | Humbert, Sebastien | |
dc.contributor.author | Rohrlich, Pierre | |
dc.contributor.author | Coaquette, Alain | |
dc.contributor.author | Vuoto, Fanny | |
dc.contributor.author | Faure, Karine | |
dc.contributor.author | Mahlaoui, Nizar | |
dc.contributor.author | Kotnik, Primoz | |
dc.contributor.author | Battelino, Tadej | |
dc.contributor.author | Podkrajšek, Katarina Trebušak | |
dc.contributor.author | Kisand, Kai | |
dc.contributor.author | Ferre, Elise M.N. | |
dc.contributor.author | DiMaggio, Thomas | |
dc.contributor.author | Rosen, Lindsey B. | |
dc.contributor.author | Burbelo, Peter D. | |
dc.contributor.author | McIntyre, Martin | |
dc.contributor.author | Casanova, Jean-Laurent | |
dc.contributor.author | Lionakis, Michail | |
dc.date.accessioned | 2022-04-20T12:03:46Z | |
dc.date.available | 2022-04-20T12:03:46Z | |
dc.date.created | 2021-11-23T10:34:31Z | |
dc.date.issued | 2021 | |
dc.identifier.issn | 0022-1007 | |
dc.identifier.uri | https://hdl.handle.net/11250/2991631 | |
dc.description.abstract | Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range of autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These auto-Abs were recently reported to account for at least 10% of cases of life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients from 21 kindreds in seven countries, aged between 8 and 48 yr and infected with SARS-CoV-2 since February 2020. The 21 patients tested had auto-Abs neutralizing IFN-α subtypes and/or IFN-ω; one had anti–IFN-β and another anti–IFN-ε, but none had anti–IFN-κ. Strikingly, 19 patients (86%) were hospitalized for COVID-19 pneumonia, including 15 (68%) admitted to an intensive care unit, 11 (50%) who required mechanical ventilation, and four (18%) who died. Ambulatory disease in three patients (14%) was possibly accounted for by prior or early specific interventions. Preexisting auto-Abs neutralizing type I IFNs in APS-1 patients confer a very high risk of life-threatening COVID-19 pneumonia at any age. | en_US |
dc.language.iso | eng | en_US |
dc.publisher | Rockefeller University Press | en_US |
dc.rights | Navngivelse-Ikkekommersiell-DelPåSammeVilkår 4.0 Internasjonal | * |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-sa/4.0/deed.no | * |
dc.title | Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1 | en_US |
dc.type | Journal article | en_US |
dc.type | Peer reviewed | en_US |
dc.description.version | publishedVersion | en_US |
dc.rights.holder | Copyright 2021 The Author(s) | en_US |
dc.source.articlenumber | e20210554 | en_US |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 2 | |
dc.identifier.doi | 10.1084/jem.20210554 | |
dc.identifier.cristin | 1957636 | |
dc.source.journal | Journal of Experimental Medicine (JEM) | en_US |
dc.relation.project | Stiftelsen Kristian Gerhard Jebsen: KGJ senter for autoimmune sykdommer | en_US |
dc.relation.project | Norges forskningsråd: 262677 | en_US |
dc.identifier.citation | Journal of Experimental Medicine (JEM). 2021, 218 (7), e20210554. | en_US |
dc.source.volume | 218 | en_US |
dc.source.issue | 7 | en_US |